Arachnodactyly

Arachnodactyly [(from Greek arachne- spider and daktylos- finger), Achard], described in 1896 by Marfan under the name dolichostenomelia, synonym - acromegaly (Pfaundler). A rare (only about 20 cases described) congenital developmental anomaly affecting almost only girls and concerning mainly the skeleton, especially the limbs. The latter are peculiarly elongated and narrowed, especially in the hands and feet (monkey hand, foot), most of all the distal phalanges, from which the similarity of the fingers to a spider's leg. The length

of the body, on the contrary, is less than normal. The chest is long and narrow. There is always a deep disturbance of nutrition and a number of other congenital defects: exostoses calcanei, dislocation of the lens and hip, webbed fingers and so on. All children with A. have a striking resemblance, later physical retardation and instability to harmful influences are noted, because of which children die early (in the described cases - the oldest was 18 years). Etiology is unknown; the assumption was made about intrauterine depletion of endocrine glands, about damage to the germ plasma. Syphilis does not play a role. The pathogenesis was sought in the intrauterine closure of epiphyseal lines (congenital acromegaly Brissaud and Meige); Mery and Babonneux speak of "hyperchondroplasia". Pathological-anatomical examination in three cases gave nothing.